Barker, Roger A, Mason, Sarah L., Harrower, Timothy P., Swain, Rachel A., Ho, Aileen K., Sahakian, Barbara J., Mathur, Raj, Elneil, Sohier, Thornton, Steven, Hurrelbrink, Carrie, Armstrong, Richard J., Tyers, Pam, Smith, Emma, Carpenter, Adrian, Piccini, Paola, Tai, Yen F., Brooks, David J., Pavese, Nicola, Watts, Colin, Pickard, John D., Rosser, Anne E., Dunnett, Stephen B.

Huntington’s disease (HD) is a fatal autosomal dominant neurodegenerative disease involving progressive motor, cognitive and behavioural decline, leading to death approximately 20 years after motor onset. The disease is characterised pathologically by an early and progressive striatal neuronal cell loss and atrophy, which has provided the rationale for first clinical trials of neural repair using […]

Vandenberghe, Wim, Demaerel, Philippe, Dom, René, Maes, Frederik

Measurement of striatal volume using magnetic resonance imaging (MRI) provides a marker of striatal degeneration in Huntington disease (HD). Recent evidence suggests that diffusion-weighted MRI (DWI) may also detect striatal damage in HD. Here, we compared the sensitivities of volumetric MRI and DWI at distinguishing 10 patients with early symptomatic HD from 12 age-matched controls. […]

Schöll, Michael, Almkvist, Ove, Axelman, Karin, Stefanova, Elka, Wall, Anders, Westman, Eric, Långström, Bengt, Lannfelt, Lars, Graff, Caroline, Nordberg, Agneta

Six young related pre-symptomatic carriers of a His163Tyr mutation in the presenilin 1 gene who will develop early onset familial Alzheimer’s disease (eoFAD), and a control group of 23 non-carriers underwent (18)F-fluorodeoxyglucose positron emission tomography (FDG PET). The mutation carriers were followed-up after 2 years. Multivariate analysis showed clear separation of carriers from non-carriers on […]

Paganini, M., Biggeri, A., Romoli, A. M., Mechi, C., Ghelli, E., Berti, V., Pradella, S., Bucciantini, S., Catelan, D., Saccardi, R., Lombardini, L., Mascalchi, M., Massacesi, L., Porfirio, B., Di Lorenzo, N., Vannelli, G. B., Gallina, P.

Objective: To assess the clinical effect of caudate-putaminal transplantation of fetal striatal tissue in Huntington’s disease (HD).; Methods: We carried out a follow-up study on 10 HD transplanted patients and 16 HD not-transplanted patients. All patients were evaluated with the Unified HD Rating Scale (UHDRS) whose change in motor, cognitive, behavioural and functional capacity total […]